PDSS1

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PDSS1
Identifiers
AliasesPDSS1, COQ1, COQ10D2, DPS, SPS, TPRT, TPT, TPT 1, hDPS1, prenyl (decaprenyl) diphosphate synthase, subunit 1, decaprenyl diphosphate synthase subunit 1, COQ1A
External IDsOMIM: 607429 MGI: 1889278 HomoloGene: 5353 GeneCards: PDSS1
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_014317
NM_001321978
NM_001321979

NM_019501
NM_001355467

RefSeq (protein)

NP_001308907
NP_001308908
NP_055132

NP_062374
NP_001342396

Location (UCSC)Chr 10: 26.7 – 26.75 MbChr 2: 22.9 – 22.94 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Decaprenyl-diphosphate synthase subunit 1 is an enzyme that in humans is encoded by the PDSS1 gene.[5][6]

The protein encoded by this gene is an enzyme that elongates the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. The gene product catalyzes the formation of all trans-polyprenyl pyrophosphates from isopentyl diphosphate in the assembly of polyisoprenoid side chains, the first step in coenzyme Q biosynthesis. The protein may be peripherally associated with the inner mitochondrial membrane, though no transit peptide has been definitively identified to date.[6]

References[]

  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000148459 - Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000026784 - Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ Rotig A, Appelkvist EL, Geromel V, Chretien D, Kadhom N, Edery P, Lebideau M, Dallner G, Munnich A, Ernster L, Rustin P (Sep 2000). "Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency". Lancet. 356 (9227): 391–395. doi:10.1016/S0140-6736(00)02531-9. PMID 10972372. S2CID 27320145.
  6. ^ a b "Entrez Gene: PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1".

Further reading[]


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